Sökning: onr:"swepub:oai:DiVA.org:umu-194848" > Characteristics of ...
Fältnamn | Indikatorer | Metadata |
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000 | 04980naa a2200529 4500 | |
001 | oai:DiVA.org:umu-194848 | |
003 | SwePub | |
008 | 220607s2022 | |||||||||||000 ||eng| | |
024 | 7 | a https://urn.kb.se/resolve?urn=urn:nbn:se:umu:diva-1948482 URI |
024 | 7 | a https://doi.org/10.1080/13506129.2022.20432702 DOI |
040 | a (SwePub)umu | |
041 | a engb eng | |
042 | 9 SwePub | |
072 | 7 | a ref2 swepub-contenttype |
072 | 7 | a art2 swepub-publicationtype |
100 | 1 | a Barroso, Fabio A.u Institute for Neurological Research, FLENI, Buenos Aires, Argentina4 aut |
245 | 1 0 | a Characteristics of patients with autonomic dysfunction in the Transthyretin Amyloidosis Outcomes Survey (THAOS) |
264 | c 2022-04-22 | |
264 | 1 | b Taylor & Francis,c 2022 |
338 | a electronic2 rdacarrier | |
520 | a Background: Autonomic dysfunction is common in transthyretin amyloidosis (ATTR amyloidosis), but its frequency, characteristics, and quality-of-life (QoL) impact are not well understood.Methods: The Transthyretin Amyloidosis Outcomes Survey (THAOS) is an ongoing, global, longitudinal survey of patients with ATTR amyloidosis, including patients with inherited (ATTRv) and wild-type (ATTRwt) disease and asymptomatic patients with TTR mutations (ClinicalTrials.gov: NCT00628745). In a descriptive analysis, characteristics and Norfolk QoL-DN total (TQoL) scores at enrolment were compared in patients with vs without autonomic dysfunction (analysis cut-off: 1 August 2020).Results: Autonomic dysfunction occurred in 1181/2922 (40.4%) symptomatic patients, and more commonly in ATTRv (1107/1181 [93.7%]) than ATTRwt (74/1181 [6.3%]) amyloidosis. Time (mean [SD]) from ATTR amyloidosis symptom onset to first autonomic dysfunction symptom was shorter in ATTRv (3.4 [5.7] years) than ATTRwt disease (9.7 [10.4]). In ATTRv disease, patients with vs without autonomic dysfunction had worse QoL (TQoL, 47.3 [33.2] vs 16.1 [18.1]); in ATTRwt disease, those with vs without autonomic dysfunction had similar QoL (23.0 [18.2] vs 19.9 [20.5]).Conclusions: Autonomic dysfunction was more common and presented earlier in symptomatic ATTRv than ATTRwt amyloidosis and adversely affected QoL in ATTRv disease. These THAOS findings may aid clinicians in diagnosing and treating patients with ATTR amyloidosis.Trial registration: ClinicalTrials.gov: NCT00628745. | |
650 | 7 | a MEDICIN OCH HÄLSOVETENSKAPx Klinisk medicinx Neurologi0 (SwePub)302072 hsv//swe |
650 | 7 | a MEDICAL AND HEALTH SCIENCESx Clinical Medicinex Neurology0 (SwePub)302072 hsv//eng |
653 | a Amyloidosis | |
653 | a autonomic nervous system | |
653 | a neuropathy | |
653 | a registry | |
653 | a transthyretin | |
700 | 1 | a Coelho, Teresau Unidade Corino Andrade, Hospital Santo António, Centro Hospitalar Universitário do Porto, Porto, Portugal4 aut |
700 | 1 | a Dispenzieri, Angelau Division of Hematology, Mayo Clinic, MN, Rochester, United States4 aut |
700 | 1 | a Conceição, Isabelu Hospital de Santa Maria–CHULN, FML Universidade de Lisboa, Lisbon, Portugal4 aut |
700 | 1 | a Waddington-Cruz, Marciau CEPARM, University Hospital, Federal University of Rio de Janeiro, Rio de Janeiro, Brazil4 aut |
700 | 1 | a Wixner, Jonasu Umeå universitet,Avdelningen för medicin4 aut0 (Swepub:umu)jowi0065 |
700 | 1 | a Maurer, Mathew S.u Columbia University College of Physicians and Surgeons, NY, New York, United States4 aut |
700 | 1 | a Rapezzi, Claudiou Cardiological Center, University of Ferrara, Ferrara, Italy; Maria Cecilia Hospital, GVM Care & Research, Cotignola (RA), Italy4 aut |
700 | 1 | a Planté-Bordeneuve, Violaineu Department of Neurology, East-Paris University, Hospital Henri Mondor, Assistance Publique Hopitaux de Paris, INSERM U955 Team 10 “Biology of the Neuro-Muscular System”, Crétei, France4 aut |
700 | 1 | a Kristen, Arnt V.u Department of Cardiology, Angiology, and Respiratory Medicine, Medical University of Heidelberg, Heidelberg, Germany4 aut |
700 | 1 | a González-Duarte, Alejandrau Instituto Nacional De Ciencias Medicas y Nutricion Salvador Zubiran, Mexico City, Mexico4 aut |
700 | 1 | a Chapman, Dougu Pfizer Inc, NY, New York, United States4 aut |
700 | 1 | a Stewart, Michelleu Pfizer Inc, CT, Groton, United States4 aut |
700 | 1 | a Amass, Leslieu Pfizer Inc, CT, Groton, United States4 aut |
710 | 2 | a Institute for Neurological Research, FLENI, Buenos Aires, Argentinab Unidade Corino Andrade, Hospital Santo António, Centro Hospitalar Universitário do Porto, Porto, Portugal4 org |
773 | 0 | t Amyloidd : Taylor & Francisg 29:3, s. 175-183q 29:3<175-183x 1350-6129x 1744-2818 |
856 | 4 | u https://doi.org/10.1080/13506129.2022.2043270y Fulltext |
856 | 4 | u https://umu.diva-portal.org/smash/get/diva2:1665360/FULLTEXT02.pdfx primaryx Raw objecty fulltext:print |
856 | 4 8 | u https://urn.kb.se/resolve?urn=urn:nbn:se:umu:diva-194848 |
856 | 4 8 | u https://doi.org/10.1080/13506129.2022.2043270 |
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